Pick disease is a brain disorder that causes slowly worsening decline of mental abilities. It gradually damages brain cells and impairs their function. It disturbs cognitive processes, such as reasoning, problem solving, and memory. The disease often affects a person’s ability to use and understand spoken, written, and even signed language. It also affects personality, emotions, and social behavior. When the decline in mental abilities is severe enough to interfere with a person’s ability to carry out everyday activities, it is called dementia. Pick disease is named after Arnold Pick, the doctor who first described the disease in 1892. It is often compared to Alzheimer disease. However, Pick disease is different from Alzheimer disease in several ways. * First, the diseases affect different parts of the brain. Pick disease usually affects only the frontal and temporal lobes of the brain, the part from the forehead back to the ears. For this reason it is sometimes called “frontotemporal dementia.” Pick disease is only one of several types of frontotemporal dementia. * * Second, the diseases damage the brain in different ways. The changes they cause in the brain are distinct. Both diseases cause severe shrinkage (atrophy) of brain tissue and death of nerve cells called neurons. In Pick disease, the neurons contain abnormal protein accumulations called Pick bodies. Neurons may swell as they stop functioning. * * These differences translate to somewhat different symptoms for the two diseases. Memory loss, usually the first symptom in Alzheimer disease, may not occur in Pick disease until later in the disease. People with Pick disease may have early changes in mood, behavior, and use of language and speech (aphasia). * * On average, Pick disease occurs at a somewhat younger age than Alzheimer disease. In Pick disease, the first symptoms typically appear in middle age, in people aged 40-60 years. However, it can occur in adults of any age. Unfortunately, Pick disease is similar to Alzheimer disease in several ways. * It is a progressive disease, meaning that the symptoms gradually worsen over time and do not get better. * * The two diseases are equally devastating, causing gradual decline of mental functions and disability. * * Neither disease is curable. Much less is known about Pick disease than about Alzheimer disease. This is partly because Pick disease is a much less common disease. Also, Pick bodies and neuron swelling are difficult to detect in a living person, so Pick disease may go undiagnosed or be misdiagnosed. People with Pick disease are sometimes thought to have Alzheimer disease. This is changing as medical professionals learn more about Pick disease.
The frontal lobes of the brain are the source of our rational emotional responses and the way we act in response to the world around us. This region of the brain also controls our use of speech and how we express language in all forms. Because Pick disease mostly affects this region of the brain, it is these thinking functions that are most often abnormal in people with the disease. The symptoms of Pick disease vary widely from person to person. The symptoms are usually very subtle at first and worsen slowly. The rate of worsening also varies from person to person. The earliest symptoms are usually changes in behavior, mood, or personality. The person may act unlike his or her usual self. The following are common behavioral changes: * Apathy (indifference) or withdrawal * * Severe depression in a person who has never been depressed before * * Blunting or dullness of emotions * * Loss of inhibition * * Impulsive behavior in a usually cautious person * * Bad manners, rudeness * * Saying or doing inappropriate things in public * * Impatience * * Becoming extroverted, very talkative * * Inappropriate joking * * Aggressiveness * * Restlessness or agitation * * Poor judgment * * Paranoia * * Selfishness * * Difficulty coping with changes from routine * * Development of obsessive routines * * Childlike behavior * * Changes in sexual behavior Other common symptoms in Pick disease include the following: * Speech problems: The person may have trouble finding the right word, may speak less, or may stop speaking altogether. His or her sentences may be incomplete or organized strangely. The person may lose the ability to understand written, spoken, or signed language. * * Changes in eating habits: The person may begin overeating, eating greedily, eating excessive amounts of sweets, or drinking large amounts of alcohol. He or she may gain weight. * * Poor attention: The person may have problems paying attention, concentrating, or carrying on a sustained conversation.
The specific cause of Pick disease is unknown. In a small number of cases, the disease is hereditary, meaning that it runs in the family. This is not true of most cases of the disease
The only way to confirm the diagnosis of Pick disease is to look at the brain directly and to identify Pick bodies and swollen neurons. This is possible only if the brain is biopsied. This means taking a small sample of brain tissue for testing. This is done by a neurosurgeon, a surgeon who specializes in operating on the brain. The sample is examined under a microscope by a pathologist, a doctor who specializes in diagnosing diseases by looking at tissues in this way. The brain can also be examined in this way at autopsy, after a person’s death. Biopsy offers a definite diagnosis while the person is still alive, and many people and their families are choosing to undergo this procedure. Without biopsy, the diagnosis in a living person is usually made on the basis of symptoms and ruling out other conditions. This is done by a combination of medical interview, physical and mental examinations, lab tests, imaging studies, and other tests. A doctor should discuss the potential risks and benefits of each approach with the patient and family. The medical interview involves detailed questions about the symptoms and how they have changed over time. Your health care provider will also ask about medical and mental problems now and in the past, family medical problems, medications taken now and in the past, work and travel history, habits, and lifestyle. A detailed physical examination is done to rule out medical problems that might cause dementia. The examination should include a mental status evaluation. This involves answering the examiner’s questions and following simple directions. In some cases, the health care provider will conduct neuropsychological testing or refer the person to a psychologist for such testing. At any point in this process, your primary care provider may refer you to a specialist in brain disorders (neurologist or psychiatrist). Neuropsychological testing Neuropsychological testing is the most accurate method of pinpointing and documenting a person’s cognitive problems and strengths. * This can help give a more accurate diagnosis of the problems and thus can help in treatment planning. * * The testing involves answering questions and performing tasks that have been carefully prepared for this purpose. The test is given by a neurologist, psychologist, or other specially trained professional. * * It addresses the individual’s appearance, mood, anxiety level, and experience of delusions or hallucinations. * * It assesses cognitive abilities such as memory, attention, orientation to time and place, use of language, and abilities to carry out various tasks and follow instructions. * * Reasoning, abstract thinking, and problem solving also are tested. Lab tests These include blood tests to rule out infections, blood disorders, chemical abnormalities, hormonal disorders, and liver or kidney problems that could cause dementia symptoms. Imaging studies Brain scans cannot detect Pick disease specifically, but they can detect atrophy and other abnormalities in the frontal and temporal lobes. The scan also helps rule out other conditions that can cause dementia. * MRI or CT scan of the brain * * Single-photon emission computed tomography (SPECT) or positron-emission tomography (PET) scan: This scans are used in certain cases when the diagnosis is doubtful. They are especially good at detecting abnormal brain function. SPECT and PET scans are available only at some large medical centers. Other tests Any of these tests may be ordered as part of the evaluation of dementia. * Electroencephalography (EEG) is a measurement of the electrical activity of the brain. It can be useful in some cases to rule out other conditions. * * Spinal tap (lumbar puncture) is a method of obtaining a sample of cerebrospinal fluid. This may be done to rule out certain other brain conditions that can cause dementia.
There is no cure for Pick disease. Treatment is aimed at improving behavior and mood problems and relieving other symptoms. For example, a speech therapist may be able to help the person improve his or her ability to communicate. Medication is helpful in many patients in easing mood and emotional difficulties. For more information, see Dementia.